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Chiari 1
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Chiari 1 malformation (CM1)
              • Synonyms

                • Arnold-Chiari 1 malformation (AC1)
              • Definitions

                • Elongated, peg-shaped cerebellar tonsils extend below foramen magnum into cervical spinal canal

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Nonvascular Interventions

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              • Image Interpretation Pearls

                                                Selected References

                                                1. Middlebrooks EH et al: Normal relationship of the cervicomedullary junction with the obex and olivary bodies: a comparison of cadaveric dissection and in vivo diffusion tensor imaging. Surg Radiol Anat. ePub, 2014
                                                2. Aghakhani N et al: Long-term follow-up of Chiari-related syringomyelia in adults: analysis of 157 surgically treated cases. Neurosurgery. 64(2):308-15; discussion 315, 2009
                                                3. Botelho RV et al: The effects of posterior fossa decompressive surgery in adult patients with Chiari malformation and sleep apnea. J Neurosurg. Epub ahead of print, 2009
                                                4. Chauvet D et al: Dura splitting decompression in Chiari type 1 malformation: clinical experience and radiological findings. Neurosurg Rev. 32(4):465-70, 2009
                                                5. Lucchetta M et al: Syringomyelia associated with Chiari I malformation. Neurol Sci. Epub ahead of print, 2009
                                                6. Struck AF et al: Idiopathic syringomyelia: phase-contrast MR of cerebrospinal fluid flow dynamics at level of foramen magnum. Radiology. 253(1):184-90, 2009
                                                7. Tisell M et al: Long-term outcome after surgery for Chiari I malformation. Acta Neurol Scand. 120(5):295-9, 2009
                                                8. Kaplan Y et al: Chronic migraine associated with the Chiari type 1 malformation. Clin Neurol Neurosurg. 110(8):818-22, 2008
                                                9. McGirt MJ et al: Symptom recurrence after suboccipital decompression for pediatric Chiari I malformation: analysis of 256 consecutive cases. Childs Nerv Syst. 24(11):1333-9, 2008
                                                10. Novegno F et al: The natural history of the Chiari Type I anomaly. J Neurosurg Pediatr. 2(3):179-87, 2008
                                                11. Piatt JH: Surgical management of the Chiari malformation Type I: the way forward. J Neurosurg Pediatr. 2(1):50-1; discussion 51, 2008
                                                12. Tubbs RS et al: Volumetric analysis of the posterior cranial fossa in a family with four generations of the Chiari malformation Type I. J Neurosurg Pediatr. 1(1):21-4, 2008
                                                13. Koç K et al: Chiari 1 malformation with syringomyelia: correlation of phase-contrast cine MR imaging and outcome. Turk Neurosurg. 17(3):183-92, 2007
                                                14. Milhorat TH et al: Tailored operative technique for Chiari type I malformation using intraoperative color Doppler ultrasonography. Neurosurgery. 53(4):899-905; discussion 905-6, 2003
                                                15. Tubbs RS et al: Surgical experience in 130 pediatric patients with Chiari I malformations. J Neurosurg. 99(2):291-6, 2003
                                                16. Eule JM et al: Chiari I malformation associated with syringomyelia and scoliosis: a twenty-year review of surgical and nonsurgical treatment in a pediatric population. Spine. 27(13):1451-5, 2002
                                                17. Genitori L et al: Chiari type I anomalies in children and adolescents: minimally invasive management in a series of 53 cases. Childs Nerv Syst. 16(10-11):707-18, 2000
                                                18. Milhorat TH et al: Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 44(5):1005-17, 1999
                                                19. Nishikawa M et al: Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg. 86(1):40-7, 1997
                                                20. Caldemeyer KS et al: Chiari I malformation: association with hypophosphatemic rickets and MR imaging appearance. Radiology. 195(3):733-8, 1995
                                                21. Menezes AH: Primary craniovertebral anomalies and the hindbrain herniation syndrome (Chiari I): data base analysis. Pediatr Neurosurg. 23(5):260-9, 1995
                                                22. Stovner LJ et al: Posterior cranial fossa dimensions in the Chiari I malformation: relation to pathogenesis and clinical presentation. Neuroradiology. 35(2):113-8, 1993
                                                23. Elster AD et al: Chiari I malformations: clinical and radiologic reappraisal. Radiology. 183(2):347-53, 1992
                                                24. Stovner LJ et al: Syringomyelia in Chiari malformation: relation to extent of cerebellar tissue herniation. Neurosurgery. 31(5):913-7; discussion 917, 1992
                                                25. Pillay PK et al: Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance. Neurosurgery. 28(5):639-45, 1991
                                                26. Barkovich AJ et al: Significance of cerebellar tonsillar position on MR. AJNR Am J Neuroradiol. 7(5):795-9, 1986
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Top Differential Diagnoses

                                                      • Pathology

                                                        • Clinical Issues

                                                          • Diagnostic Checklist

                                                            TERMINOLOGY

                                                            • Abbreviations

                                                              • Chiari 1 malformation (CM1)
                                                            • Synonyms

                                                              • Arnold-Chiari 1 malformation (AC1)
                                                            • Definitions

                                                              • Elongated, peg-shaped cerebellar tonsils extend below foramen magnum into cervical spinal canal

                                                            IMAGING

                                                            • General Features

                                                              • Radiographic Findings

                                                                • CT Findings

                                                                  • MR Findings

                                                                    • Nonvascular Interventions

                                                                      • Imaging Recommendations

                                                                        DIFFERENTIAL DIAGNOSIS

                                                                          PATHOLOGY

                                                                          • General Features

                                                                            • Staging, Grading, & Classification

                                                                              • Gross Pathologic & Surgical Features

                                                                                • Microscopic Features

                                                                                  CLINICAL ISSUES

                                                                                  • Presentation

                                                                                    • Demographics

                                                                                      • Natural History & Prognosis

                                                                                        • Treatment

                                                                                          DIAGNOSTIC CHECKLIST

                                                                                          • Consider

                                                                                            • Image Interpretation Pearls

                                                                                              Selected References

                                                                                              1. Middlebrooks EH et al: Normal relationship of the cervicomedullary junction with the obex and olivary bodies: a comparison of cadaveric dissection and in vivo diffusion tensor imaging. Surg Radiol Anat. ePub, 2014
                                                                                              2. Aghakhani N et al: Long-term follow-up of Chiari-related syringomyelia in adults: analysis of 157 surgically treated cases. Neurosurgery. 64(2):308-15; discussion 315, 2009
                                                                                              3. Botelho RV et al: The effects of posterior fossa decompressive surgery in adult patients with Chiari malformation and sleep apnea. J Neurosurg. Epub ahead of print, 2009
                                                                                              4. Chauvet D et al: Dura splitting decompression in Chiari type 1 malformation: clinical experience and radiological findings. Neurosurg Rev. 32(4):465-70, 2009
                                                                                              5. Lucchetta M et al: Syringomyelia associated with Chiari I malformation. Neurol Sci. Epub ahead of print, 2009
                                                                                              6. Struck AF et al: Idiopathic syringomyelia: phase-contrast MR of cerebrospinal fluid flow dynamics at level of foramen magnum. Radiology. 253(1):184-90, 2009
                                                                                              7. Tisell M et al: Long-term outcome after surgery for Chiari I malformation. Acta Neurol Scand. 120(5):295-9, 2009
                                                                                              8. Kaplan Y et al: Chronic migraine associated with the Chiari type 1 malformation. Clin Neurol Neurosurg. 110(8):818-22, 2008
                                                                                              9. McGirt MJ et al: Symptom recurrence after suboccipital decompression for pediatric Chiari I malformation: analysis of 256 consecutive cases. Childs Nerv Syst. 24(11):1333-9, 2008
                                                                                              10. Novegno F et al: The natural history of the Chiari Type I anomaly. J Neurosurg Pediatr. 2(3):179-87, 2008
                                                                                              11. Piatt JH: Surgical management of the Chiari malformation Type I: the way forward. J Neurosurg Pediatr. 2(1):50-1; discussion 51, 2008
                                                                                              12. Tubbs RS et al: Volumetric analysis of the posterior cranial fossa in a family with four generations of the Chiari malformation Type I. J Neurosurg Pediatr. 1(1):21-4, 2008
                                                                                              13. Koç K et al: Chiari 1 malformation with syringomyelia: correlation of phase-contrast cine MR imaging and outcome. Turk Neurosurg. 17(3):183-92, 2007
                                                                                              14. Milhorat TH et al: Tailored operative technique for Chiari type I malformation using intraoperative color Doppler ultrasonography. Neurosurgery. 53(4):899-905; discussion 905-6, 2003
                                                                                              15. Tubbs RS et al: Surgical experience in 130 pediatric patients with Chiari I malformations. J Neurosurg. 99(2):291-6, 2003
                                                                                              16. Eule JM et al: Chiari I malformation associated with syringomyelia and scoliosis: a twenty-year review of surgical and nonsurgical treatment in a pediatric population. Spine. 27(13):1451-5, 2002
                                                                                              17. Genitori L et al: Chiari type I anomalies in children and adolescents: minimally invasive management in a series of 53 cases. Childs Nerv Syst. 16(10-11):707-18, 2000
                                                                                              18. Milhorat TH et al: Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 44(5):1005-17, 1999
                                                                                              19. Nishikawa M et al: Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg. 86(1):40-7, 1997
                                                                                              20. Caldemeyer KS et al: Chiari I malformation: association with hypophosphatemic rickets and MR imaging appearance. Radiology. 195(3):733-8, 1995
                                                                                              21. Menezes AH: Primary craniovertebral anomalies and the hindbrain herniation syndrome (Chiari I): data base analysis. Pediatr Neurosurg. 23(5):260-9, 1995
                                                                                              22. Stovner LJ et al: Posterior cranial fossa dimensions in the Chiari I malformation: relation to pathogenesis and clinical presentation. Neuroradiology. 35(2):113-8, 1993
                                                                                              23. Elster AD et al: Chiari I malformations: clinical and radiologic reappraisal. Radiology. 183(2):347-53, 1992
                                                                                              24. Stovner LJ et al: Syringomyelia in Chiari malformation: relation to extent of cerebellar tissue herniation. Neurosurgery. 31(5):913-7; discussion 917, 1992
                                                                                              25. Pillay PK et al: Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance. Neurosurgery. 28(5):639-45, 1991
                                                                                              26. Barkovich AJ et al: Significance of cerebellar tonsillar position on MR. AJNR Am J Neuroradiol. 7(5):795-9, 1986